BACKGROUND: Fungal endocarditis in neonates, often caused by Candida albicans, is a rare but severe condition linked to invasive medical interventions. Preterm infants are particularly vulnerable due to their immature immune systems. Diagnosis is challenging due to nonspecific symptoms, and management typically requires both antifungal therapy and surgery. CASE REPORT: A 43-day-old preterm female infant, born at 34 weeks with intrauterine growth restriction, presented with respiratory distress. She had been treated for suspected sepsis and found to have large tricuspid vegetations. Blood cultures confirmed Candida albicans. Despite initial antifungal treatment, surgery was performed due to the size of the vegetations. After surgery, the patient was treated with caspofungin, leading to clinical improvement. At 9-month follow-up, she showed full recovery with normal cardiac function. CONCLUSION: This case demonstrates the successful management of neonatal fungal endocarditis through early diagnosis, surgical intervention, and appropriate antifungal therapy, leading to full recovery in a preterm neonate.